Alveolar Rhabdomyosarcoma: a rare entity of tumor of the paranasal sinuses in adults, a case report

Rhabdomyosarcoma are the most common soft-tissue malignancy of childhood, but rarely occur in adults. We report about a 63-year-old man with progressive right nasal obstruction and a swelling of the right cheek over a few weeks. The right nasal passage was completely blocked and the cheek was bulged out by a subcutaneous tumor, moreover there were right cervical lymphadenopathies noticed. Motility of the eye was normal. CT and MRI scans showed a mass of the right nasal cavity and paranasal sinuses with infiltration of the orbit, facial subcutis and the frontal base of the scull furthermore an intracranial extension of the tumor via V2. Multiple parotideal and cervical nodal metastases were noticed but no hint for distant metastases. After extensive immunhistochemical and molecularpathological examinations of the biopsy, the most likely diagnosis was an undifferentiated carcinoma. A neoadjuvant chemotherapy with Etoposid/Cisplatin followed by radiochemotherapy was conducted. Already after 3 months, the patient presented with decrease of general condition, anemia and thrombocytopenia. By examination of the bone marrow eventually, diagnosis of alveolar Rhabdomyosarcoma was made. This also was confirmed in the biopsy of the paranasal sinus. General condition deteriorated noticeably under chemotherapy with Adriamycin and limitation of therapy in terms of best supportive care was concerted. Alveolar Rhabdomyosarcoma of the nasal cavity and paranasal sinuses is a rare differential diagnosis in adults. To some extent, diagnosis might be difficult and might lead to a fatal delay for the patient.

Publication History

Article published online:
12 May 2023